We are very lucky to have Victoria Dawes, a pelvic floor physiotherapist from Toronto and grandmother of a child with CF, write an article for us on a topic that is not very often discussed but impacts many of our lives. This is urinary incontinence as a result of hard coughing, and can be especially problematic for women. I am so grateful to Victoria for putting together this fantastic information for us, and some exercises we can use to address the problem.

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I COUGHED SO HARD I PEED MY PANTSby Victoria Dawes, Registered Physiotherapist Under ordinary circumstances , with the stress and strain of daily life, one in four women will develop urinary incontinence at some point in her life because of pelvic floor muscle dysfunction. Women with Cystic Fibrosis who have a persistent cough are at even higher risk because of the increase in abdominal pressure during coughing which displaces the pelvic floor downward. Knowing how to effectively engage the pelvic floor muscles will not only prevent leakage of urine but will create a more powerful cough to clear airway secretions/mucous because the force of the cough will be directed upward as the strong pelvic floor muscles resist the increase in abdominal pressure. Coughing is exhausting so it is desirable to develop a strong, effective cough and at the same time prevent embarrassing urine leaks.

The pelvic floor is a deep, dark mysterious place. The biggest challenge with pelvic floor activation is that many of us do not have awareness or perception of the pelvic floor muscles. In other words, it is very difficult to train muscles that we can’t easily see, touch, or visualize. Rehabilitation of the pelvic floor is as much about rewiring the nervous system to develop communication between the brain and the perineum as it is about strengthening the muscles. The pelvic floor has two opposite functions: it is closed to provide support for the organs and prevent escape of urine and stool, but needs to open up for urination, defecation, intercourse and childbirth. The pelvic floor plays a role in core stability, breathing, balance, posture, body centering, and facilitation of blood and lymph circulation in the pelvic basin. The pelvic floor is a hammock of layered muscles that stretches from the pubic bone at the front and attaches to the sacrum and tailbone at the back. It forms a soft bottom to the abdomen. We can stop the flow of urine by contracting the front of the pelvic floor, and stop the passing of gas by contracting the back of the pelvic floor. Although the entire pelvic floor generally works as a unit we can isolate separate areas. Visualization is a powerful tool that can be used to recruit the muscles of the pelvic floor. By focusing on imagery we can develop awareness of the perineum and greatly improve the function. The pelvic floor muscles are programmed to work along with the respiratory diaphragm so when first learning a Kegel it is best to contract the pelvic floor muscles as you breathe OUT, and relax the muscles as you breathe IN. A healthy muscle needs to be able to relax and have flexibility as much as it needs to have power and endurance.

LEARNING HOW TO DO KEGEL EXERCISES

Imagine that there is a marble just outside your anus and you are drawing it into your rectum. Hold the contraction while you breathe out ( 5 seconds) then relax as you breathe in and rest for another breath ( 15 sec) . Repeat the contract/relax sequence 5 times.

Imagine that the sitz bones (the bones you feel in your buttocks when you are sitting) are magnets. As you breathe out visualize the magnets being strongly drawn together. As you breathe in relax then rest. Repeat as in #1

Imagine a ping pong ball sitting just outside the vagina. Visualize squeezing the ball and drawing it into your vagina as you breathe out. Relax as you breathe in and relax then rest. Repeat the sequence as in #1.

Picture that you are picking up a jellybean with your labia as you breathe out. Relax as you breathe in, then rest. Repeat the sequence 5x.

Imagine that you are drawing a raisin into your urethra or that you are lifting the urethra up behind the pubic bone. Repeat as above.

When you have developed an awareness of the separate areas you can begin to combine the effort in various combinations ie. # 1+ 2, # 2 + 3, # 3 + 4, # 4 + 5

Now put it all together: as you breathe out engage the entire pelvic floor by imagining an elevator rising, or a hammock being pulled out and lifted, or a lotus flower tightly closed. On the relaxation phase visualize the elevator being lowered to the sub-basement, the hammock lowered and slack, or the lotus flower completely opened out.

It is also important to exercise the fast-acting muscle fibres that quickly engage in response to the urge to cough, sneeze, and on impact. Contract for 1 second, then immediately relax for 1 second. Repeat 10 x. Visualize the action like a ball bouncing, i.e. it has to hit the ground before rising up again.

Finally, as you begin to breathe out engage the entire pelvic floor and maintain the hold while you produce one strong cough. Relax and rest after the cough then repeat 5x.

Exercises 1 - 7 develop the muscles that provide endurance and support to the organs and sphincters. Exercises 8 - 9 strengthen the response to prevent urine leaks during coughs and are important in sexual function. REPEAT THE EXERCISES SEVERAL TIMES PER DAY AND AFTER SEVERAL MONTHS YOU WILL SEE AN IMPROVEMENT IN THE STRENGTH AND SUPPORT OF THE PELVIC FLOOR MUSCLES. Be diligent and purposeful in your practice. Engaging these muscles requires concentration but with some effort your brain will learn how to use them effectively again. If you are not getting stronger, or this is too abstract to learn on your own, and it is within your means, seek treatment from a Pelvic Health Physiotherapist (Physical Therapist) who has specialized training to help you retrain the pelvic floor muscles using internal palpation. You can search for a qualified P.T. in Canada at www.pelvichealthsolutions.ca Or on www.physiotherapy.ca/Find-a-Physiotherapist or in the United States on the American Physical Therapy Website under Women’s Health. www.women’shealthapta.org/plp/ You can direct any questions to the author of this information: Victoria Dawes Registered Physiotherapist, Women’s Pelvic Healthv.dawes@pelvicfloorplan.com

The emerging understanding of the critical role that microbes play in our health is going to revolutionize the way we understand disease, and turn modern medicine on its head. Below is an article from my favorite science magazine, New Scientist.

Why We Need Ecological MedicineWe know which species make us sick, but we must learn which species make us healthy.By Rob DunnIt was a revelation. Germs cause disease. When Louis Pasteur and Robert Koch discovered and developed what would later be called the germ theory in the 1860s, this was a radical, then revolutionary idea—one so good it seems obvious in retrospect.

At the heart of their work was the notion that individual species cause disease by invading our bodies. Over the next century, the notion of "germs" changed our behavior. It led us to scrub our hands and actively fight specific pathogens (as researchers came to call dangerous germs) and to cure the diseases they cause. These changes saved millions, maybe billions of lives. Every day you rub shoulders with the success of this theory. How could there be anything wrong with it?

New research, however, is beginning to question, if not germ theory itself, at least some of the actions we have taken on its behalf. These studies come from very different groups of scientists, largely working separately and apparently without much awareness of one another. But I believe that they are unwittingly part of the slow unraveling of a new, broader theory of disease, the ecological theory of disease.

Here's the thinking. In the late 1980s, microbiologists and public-health researchers began to notice differences between rural and urban kids. Rural kids seemed less likely to develop allergies. A new idea was floated—perhaps they had been exposed to more bacteria that had helped their immune systems to "balance" themselves. This idea, often called the hygiene hypothesis, has since found support in empirical studies worldwide.

Country kids whose fingers still plunge regularly into the rich bacteria of soil (and farm animals) have fewer allergies. But it isn't just farm living: Sometimes the exposure to a wilder bacterial life can be subtle. For example, a recent study in Australia found that pregnant mothers living with dogs were less likely to have children with allergies. These studies note fundamental differences between the immune systems of dirty kids and clean kids. Conclusion: In some ways it is better to be dirty.

More recently, a new version of the hygiene hypothesis has suggested that it isn't just large numbers of bacteria that it is good to be exposed to but, rather, many kinds of bacteria. Our immune system needs to be exposed to many species in order to sort the good from the bad. Without such exposure, argues this "biodiversity" version, mistakes get made. The immune system, in not having seen enough of the world, doesn't know quite what to attack. It attacks pollen. It attacks us.

This made me sit up and take notice. There are, I realized, many separate fields of science in which the failure to be exposed to good species or even just a diversity of species is believed to make us sick.

The "worm hypothesis" argues that our bodies evolved with parasitic worms as a dependable presence, and that for some individuals the absence of such worms causes the immune system to overreact, leading to autoimmune diseases such as Crohn's, multiple sclerosis, and asthma. The nature deficit hypothesis, on the other hand, argues that lack of exposure to nature in our city environments causes psychological problems in children who then suffer from any of a variety of behavioral and other problems. This is country cousin to the biophilia hypothesis, which suggests an innate fondness for nature and biodiversity, which both bring us benefits and, in their absence, costs.

All of these relate to the much older and well-accepted "deficiency" model, which correctly states that diseases such as scurvy are caused by the absence of whole classes of species (and their nutrients) in our diets.

What seems to have gone pretty much unremarked is that these ideas all suggest ways in which the absence of beneficial or historically common species in our lives can make us sick. In a way, taken together these ideas make up the obverse of the germ theory of disease; if the germ theory is about bad species being present, these hypotheses are all about good species that have gone missing.Bringing the pieces of the puzzle together seems to show what I call the ecological theory of disease. This is the idea that illness can arise from the presence of species that negatively affect our health or the absence of species that positively affect our health.Of course, to ecologists and evolutionary biologists, such a theory is not exactly news. We can all hold up long lists of species that require other species, their partners and neighbors, to survive. Think corals, lichens, leaf-cutter ants, tube worms, bean plants. Now think humans. Take away the species we benefit from every day and we would die in many different ways.The point is that public-health researchers, medical researchers and doctors don't think like ecologists. Hospitals only consider other species when they are "bad," when, that is, they are behaving as germs. With a couple of examples we tend to regard as freakish (the medical use of leeches or fly maggots), doctors almost never prescribe the apple, bacteria, worm, or other sort of "nature" your body is "missing," though if you took just the right mix it would surely help keep the doctor away.So what should we do? If the germ theory of disease tells us to hunt down, scrub off and otherwise avoid bad species, the ecological theory of disease suggests the same, but that we also need to figure out how to attract, farm, and nurture beneficial species. Fine. But there is a big problem: While we have spent the last 200 years chasing down bad species, we have spent far less time hunting good ones. Worse, while there are hundreds of pathogens that affect our health and well-being (with a small handful being the really deadly monsters), the precise mélange of beneficial species we need could involve hundreds of thousands of species—or more.Those species do not always have names. Recently, I cataloged the species on my body and my house, finding more than 2,000 species, most of which most experts could not identify. Which ones were good for me? Who knows? What is worse, no one could tell me which good species I might be missing.More and more, we seem to "know" that we need nature. Many of its species benefit us, but we are not yet smart enough to know which ones. We are left to wait for the systematists—those catalogers of life—to find and name the species on our behalf. And then we will have to wait some more for the ecologists and evolutionary biologists to study those species. Only then, finally, will medical researchers begin to weigh up which ones we need and which ones we don't. But it will take a while.We have neglected the book of life for so long that at our current rate of research, without investment in projects larger than any yet imagined, much less implemented, we won't catch up for hundreds of years. Meanwhile, some of the species we are losing from forests and wild lands (or just from our modern lives) could easily be the ones that help to make us whole.If we only knew which ones.This article originally appeared in New Scientist.Rob Dunn is the author of The Wild Life of Our Bodies, the story of our changing relationship with predators, parasites, mutualists, commensals, and all the rest. He is a science writer and scientist at North Carolina State University, where he studies the stories of the species that have lived alongside humans as we have spread around the world, be they bacteria in your belly buttonants in your backyard or cave crickets in your basement.

Because I've been so focused on presenting the research and medical discoveries that I've come across through this website, I have neglected to write about possibly the most important part of my healing regime: exercise. My whole journey to self-healing started when I got serious about exercise. One day I realized that exercise is not just something my docs tell me to do (that's probably why I was resistant for so long, actually); it is truly a powerful physical treatment and a spiritual cleansing. For me, exercise means being outdoors, challenging my body to discover its own potential, clearing my mind of the day's thoughts and worries, and becoming quiet enough to listen to the innate wisdom of my body and of nature.

Since becoming very strict about my exercise regime, which consists of at least an hour of hiking, yoga, or jogging everyday (plus biking and walking everywhere - I hardly ever use a car), my disease has been radically altered. Before I started this routine, I was physically weak and would huff and puff up a flight of stairs, get exhausted after a short walk, and was fatigued pretty much all the time. At the beginning of my new exercise routine, my FEV1 jumped almost 10% in a month! Now, even though my FEV1 has declined somewhat, I am stronger and more fit than I have been in years. I can do more rigorous physical activity for longer periods of time than I could when my FEV1 was higher. In this way, the numbers have little bearing on my quality of life. I wanted to make up t-shirts that say, "Screw the numbers, I feel great!". Truly, I care a hell of a lot less about my FEV1 than I do about how my lungs feel, how strong I am, and the quality of my emotional life. Of course, I am trying to increase my FEV1, and most likely it would have dropped a lot more in the last year and a half if I wasn't exercising so regularly. The stronger my muscles get, the more efficient they become at using energy and oxygen, so I can do more and go farther on less. I am tired much less often now, which also has a lot to do with the improvements in my diet that I've made.

I also feel that the muscles and tissue in my lungs themselves are healthier from so much stimulation and increased blood flow. Although my FEV1 has declined, my CT-scans show little change in my bronchiectasis, and even a slight decrease in the bronchiectasis in the upper lungs! I believe this has everything to do with how exercise helps me clear out the mucus, increase the blood flow to my lung tissue, and improve the elasticity of my airways through constant movement (remember, bronchiectasis is a loss of airway elasticity). My posture has also improved, and my chronic low back pain has pretty much disappeared (yoga is wonderful for this).

In addition, exercise improves insulin sensitivity. Physical exertion burns up the sugar stored in your cells to fuel your body. When that sugar is used up, the cells need to restore their supply, so they upregulate the number of insulin receptors on the cell membrane so that insulin can push more sugar from the blood into the cells, reducing our blood sugar. This is another reason why we need to exercise regularly - to maintain insulin sensitivity and to keep our blood sugar under control. So exercising when we're "sick" and plugged up with mucus is very important not only to move all that junk out through coughing, but also to increase our insulin sensitivity, which can decline when we are stressed or have an infection.

In Chinese medicine exercise is considered to facilitate proper digestion, as it balances the liver-gallbladder complex which also governs emotional health. Furthermore, with damp conditions involving a lot of mucus, exercise is considered critical to their treatment as it introduces "wind" into the body, drying up the dampness as a breeze dries a wet cloth that hangs out to dry. When I am feeling lethargic and tired, instead of napping which usually makes the situation worse, I go for a hike. Even something as simple as walking or hiking builds chi in the body, clearing away the chemicals and energetic stagnation that makes us feel tired. In Ayurveda, it is understood that our bodily energy comes from the prana, which mean "life force" and enters us through the breath. Without fail, I feel more energized and alert at the end of every hike, even if at the beginning I felt like I wanted to curl up in the ferns on the side of the trail and take a nap. Building chi through exercise is a very effective way for me to boost my energy and fight fatigue.

But the best part about exercising is the mental and physical satisfaction that I get from being outside in nature and doing something to nurture myself. I don't do exercise because my docs tell me to. I do it because I love it, and because it is helping me on my spiritual path. And I can clearly feel the results. For me, exercise is also a practice in cultivating greater awareness of my thoughts and emotions. Through movement, I become more human, more at one with the energies that I create and that surround and move through me.

And there are so many ways to exercise; so may activities to choose from! From walking to biking, horseback riding, dancing, rock climbing, running, snowboarding, sledding, yoga, working out, hiking, martial arts, gardening, swimming, ice skating... basically anything that moves your body! These things are also known as "fun", by the way. The human body was designed to exercise, either gently or rigorously, all day everyday. Even standing up or walking from one side of the room to the other is healthier than sitting at a desk for hours. This is true for humans in general, but especially for CFers who can't afford to let that mucus pool in the lungs for too long. As my friend Colin tells me, "Let's get the gravy out!".

Exercise was what started my journey towards full empowerment. Exercise allowed me to see the effects of taking full control of and responsibility for my life. I realized that healing is my responsibility. No one is going to do it for me. If I want healing, then I have to do it myself. Period. This realization gave me back such a sense of power, something I had lost after so many years of victimizing myself and being psychologically dependent on other people to "take care of me". It became clear that my life is what I make of it, and if I want to be happy and healthy, then all I have to do is make an effort to manifest it and believe that I am happy and healthy. Health can be defined in so may ways, but to me, it means living life fully and with great awareness of myself and how I move through the world. Can health really be defined by numbers? No, I don't think so. Health is a state of mind-body-spiritual balance. Can we be healthy even when our numbers are low and when all the test results come out "poor"? Hell yes! Health is a state of mind. I have met plenty of people who are free from "disease" but that I would not call healthy because their emotional, mental, and spiritual lives were out of balance and unsatisfying to them. It's obvious that there's much more to us than our cells and organs, so having a more holistic, non-materialistic view of health and wellbeing is very helpful to me in living a happy life.

Anyway, I could go on and on philosophizing, but the main point is that exercise is not only critical for maintaining physical health, but it also helps us become more complete, happy human beings through the fulfillment of our full potential.

I also want to mention the Cystic Fibrosis Lifestyle Foundation, a national organization that gives grants to people with CF to pay for exercise opportunities that may be financially out of reach otherwise. I get a scholarship from them to go to yoga. Don't let money get in the way of exercising! If you think you could benefit from an exercise grant, please check out CFLF and send in your application (it's really simple and your chances of receiving a grant are very high). Also consider donating to the organization if you're able!

For those of you who have already read CF201 and don't want to dig through the whole page once again to find my new material, here are (most of) the sections that I have added/updated:

Bronchiectasis and InflammationCF is described as a classic example of chronic inflammatory disease. Bronchiectasis, the main constituent of CF lung disease, is the result of out-of-control inflammation. It is described as the over-dilation of our airways to the point where they lose their elasticity and scarify. This causes the airways to become fragile and increases the risk of them collapsing or breaking, which can lead to hemoptysis (bleeding of the bronchial arteries into the airways leading to coughing up of blood). Our airways over-dilate in part because they are filled with mucus, and in order to move any air through a clogged up airway, they must widen themselves. But bronchiectasis is also caused by neutrophilic inflammation and the airway scarification and restructuring that results.

The inflammatory processes that cause bronchiectasis are complicated and not fully understood, but much of the problem has to do with over-recruitment of neutrophils into the airways. Neutrophils are white blood cells that kill pathogens and help clean up infection. Under normal circumstances, these guys are wonderful and target infection quickly, clean it up, then move on. But in situations of chronic infection, neutrophils (NTs) never really move on. In CF the situation is even more complex. It turns out that CF lungs have higher than normal levels of NT elastase (an enzyme that NTs use to kill pathogens and break down tissue) even without pathogenic colonization [23,24]. We don't know why this is for sure, but I postulate that one possibility could be autoimmunity. In fact, cases of non-CF bronchiectasis in older Americans are increasing at a rate of about 8.7% per year, and it is often diagnosed in patients with autoimmune diseases, especially rheumatoid arthritis [24]. Regardless, neutrophils and the inflammatory mediators that they secrete (e.g. NT elastase, IL-8) cause further neutrophil recruitment, tissue damage, and airway remodeling (i.e. bronchiectasis) [23]. It has been found that the greater the level of NT elastase in CF sputum, the lower a person's FEV1 will be [25]. Not only does NT elastase break down the elastin, collagen, and protoeoglycans in the tissue of our airways, but it also makes our mucus thicker and harder to expel. This is a problem because thick mucus provides an even better home for pathogens to breed, plus it makes it harder for the NTs to move around in the airways, so they get stuck, die, and cause mucus pooling, which causes the airways to widen even more to get around the mucus pooling, causing further bronchiectasis. Pulmozyme (DNAase) works by breaking apart neutrophil elastase in the airways, making the mucus thinner and easier to cough out.

Another problem with neutrophilic inflammation in CF is that although the NTs are called in to kill pathogens, they don't actually do a very good job because they are lacking in the antioxidant glutathione - the number one most important tool that NTs use to kill pathogens. It is thought that CF cells have a hard time secreting glutathione into intercellular space because of the faulty CFTR channel, so NTs have less access to this important antioxidant. Several studies have looked into reducing systemic inflammation by boosting NT glutathione levels with NAC supplementation. N-acetylcystine (NAC) is a molecule necessary for the synthesis of glutathione in the body, otherwise called a glutathione precursor. Direct supplementation with glutathione is largely ineffective because it is easily oxidized by environmental factors. For years NAC has been used to boost glutathione in the lungs of CF patients by inhaling it in a prescription called "Mucomyst". This was effective, but went out of fashion as pulmozyme came along, plus I've heard that mucomyst wasn't so popular because it had a rotten egg smell when aerosolized. Now there is more research suggesting that oral supplementation with NAC may also be effective in boosting NT glutathione in the body as a whole and in the lungs in particular, making our immune system more effective at clearing infection. In a preliminary study, oral NAC supplementationsignificantly boosted NT glutathione and blood glutathione in the whole body, significantly reduced the number of NTs in the airways, significantly reduced IL-8 (an inflammatory marker) in the airways, and significantly reduced NT elastase in the sputum [25]. This is a big deal! This means that instead of dealing with systemic inflammation through palliative treatments like steroids, high-dose ibuprofen, and azithromycin, NAC supplementation targets the root cause of the problem with a completely harmless treatment that has virtually no side-effects and no known level of toxicity! A larger clinical study has just been concluded on the effect of NAC on CF lungs, and I look forward to exploring the results when they're published. The standard dose for oral NAC is 600 mg three times a day, and you can ask your doctor for a prescription for it. It is used in modern medicine to treat tylenol poisoning and interstitial lung disease, so your doc should be able to get your insurance company to cover it no problem. If not, you can also get it over the counter. There's a lot of interest in the CF community in a new over the counter form of it called PharmaNAC.

Given all this, I have decided to focus on improving my lung symptoms by targeting inflammation as its main cause. I have struggled with bouts of significant hemoptysis in the last several years, and had my first pulmonary embolism in May. I have noticed that I get hemoptysis when I am particularly inflamed, caused either by a bad lung infection, inflammatory foods, and/or environmental irritants (e.g. campfire smoke or heavy pollen). Thus, in addition to adhering to the GAPS diet, I got my doc to give me a prescription for NAC, and we'll see if this improves things. In addition, to treat systemic inflammation one must consider the role of gut inflammation, the cause of most inflammatory disease. Targeting the root cause of my systemic inflammation by focusing on the gut through improving my diet and cultivating a more balanced intestinal bacteria community has yielded very positive results so far, and I'm only in the very beginning of this treatment regime, so more progress is sure to come.

Your Gut is Your Immune SystemOne way that your gut acts as part of your immune system is that the latter part of your small intestine, called the ileum, is lined with tissue called lymph nodes which kill and remove bacteria, viruses, fungi, and dead cells from the lymph (the fluid that circulates through the lymphatic system, exists in the spaces between organs, and interacts with the blood). In this way your ileum is a direct part of the immune system, and its ability to remove and kill pathogens in the interstitial space and the blood is critical to our health. When an infection is detected, the lymph nodes create lymphocytes which kill the pathogens, causing pain and inflammation in the lymph nodes. This means that if the lymph nodes in the gut are chronically exposed to toxins and pathogens, or if they have trapped pathogens within the nodes but can't clear them out, the ileum can become inflamed and painful causing serious digestive upset, reduced absorption capacity, and compromised immunity [28]. In addition, your body is an ecosystem that harbors over 400 species of bacteria just in the intestines themselves. But the whole body is populated by bacteria; the skin, eyes, nose, mouth, ears, genitals, lungs, etc. In fact, you have more bacterial cells in and on your body than you do human cells! In reality we are more bacteria than human. We are walking, talking ecosystems, and our bacteria keep us happy and healthy... if we keep them happy and healthy. Beneficial bacteria in the gut play a critical role in our immune system, keep pathogenic bacteria in check, help us break down and digest foods, and synthesize a number of nutrients that we cannot create ourselves including vitamin K2, folic acid, vitamins B1, B2, B3, B6, B12, and various other amino acids [28]. But in with our modern toxic lifestyles, it is becoming harder and harder to keep our beneficial bacteria healthy and our internal ecosystem balanced. Toxic foods, environmental pollutants, pesticides, antibiotics, pharmaceutical drugs, chronic stress, lack of exercise and time outdoors in the dirt, and many other factors have very negative effects on our bacterial populations, especially in the G.I. tract. For CFers, we have an added stressor to our inner-ecosystem, and that is our genetic defect of the CFTR channel. This makes it easier for certain pathogenic bacteria, viruses, and fungi to take hold and create an ecological imbalance.

This is particularly true in the CF digestive system. Recent research has found that the bacteria that inhabit the gut early in life determines, in part, the bacteria that inhabit the lungs later in life. (As a side note, breast-feeding ensures an infant develops a healthy and diverse intestinal flora, so breastfeed your babe as long as possible!). The healthier the intestinal ecosystem is, the more resilient the lungs are to pathogenic bacterial infection. Thus, the guts and lungs are directly connected via their bacterial populations: "these findings are consistent with previous reports, with identification of bacteria in the respiratory tract in CF that are typically associated with the intestinal cavity and are theorized to contribute to the continuum of interactions between the host and microbial community in CF that relates to both the lung and gut microbiota" [26]. Although the biota of the guts and lungs are different, many species are shared between them, and this is especially true of pathogenic bacteria, like staph. Furthermore, it has been found that respiratory bacterial populations are not particularly diverse in healthy lungs, however the less diverse the CF lung is, the more severe the respiratory disease and the lower the lung function. And the less diverse the intestinal flora, the less diverse the lung flora will be as a result. In other words, the more species you have in your lungs and guts, the better your lung functions will be. This is very similar to measuring the health of an ecosystem (like a rainforest) by its biodiversity, i.e. the number of species that inhabit it. A decline in CF lung microbiome diversity is associated with antibiotic use, therefore antibiotic use is at least partially responsible for declining lung function: "[research has] identified that diversity decreased over time in parallel with progressive disease and remained stable in patients with milder lung disease; however, they identified antibiotic use rather than lung function as the most significant driver of decreased microbial diversity in sputum samples. Additionally, based on sputum samples, Stressmann et al. corroborated similar findings for 14 patients with CF that antibiotic use was most associated with decreased microbial diversity and that overall decreased diversity was correlated to more-severe lung disease, as well as abundance of pseudomonas aeruginosa" [26]. Thus, it is very important for us to use antibiotics sparingly and only when necessary, as they are particularly detrimental to the intestinal bacteria that keep us healthy and protect our lungs from further infection and domination by only a handful of pathogenic species. Plus, emerging research is showing that even one short-term round of antibiotics can permanently impact the intestinal ecosystem and reduce its biodiversity. What does that mean for CFers who have been on regular courses of high-dose antibiotics their whole lives? An intensely disturbed intestinal ecosystem and a high risk for all of the problems that come along with it (especially leaky gut syndrome). Inhaled antibiotics do not impact intestinal flora, and certain IV antibiotics like vancomycin also have minimal impact. But all oral antibiotics impact gut flora, and most IV antibiotics do as well (tobramycin, meropenum, ceftazadime, linezolid, etc.). Unfortunately, most CF docs don't know about this problem so they go on prescribing us antibiotics in complete ignorance of what they are doing to our guts and our immune system. They are pulmonologists by training, and allopathic medicine generally sees organ systems as separate and independent from one another. The latest research is blowing this last belief apart. So don't be discouraged if your doc doesn't know any of this. They were not trained in it, so it is up to us to educate them.

Even more exciting is the research being done on treatment of the lungs through supplementation with oral probiotics. One study found that oral supplementation with only a single strain of Lactobacillus (GG) significantly increased CF childrens' FEV1, increased body weight, reduced the number and duration of hospitalizations, reduced the number of pulmonary exacerbations, and reduced the number of inflammatory markers (IgG). LGG and other Lactobacillus strains have a direct effect against Pseudomonas and have a systemic anti-inflammatory effect [27]. In conclusion, taking oral probiotics is extremely important for CFers, not only to restore intestinal microbial diversity, but to fight systemic inflammation and lung infections as well. But in order to restore ecological diversity in the intestines and the lungs, we should choose probiotics with as many strains of beneficial bacteria as possible. I discuss the use of probiotics more in my Supplements and Herbs section. Gut Dysbiosis and Carbohydrate Malabsorption The fundamental problem is that due to a number of factors including inheritance of your parents' intestinal bacteria (through the birth canal, breast feeding, and daily interaction), antibiotic or other pharmaceutical use, stress, poor diet, and pollution you have an intestinal ecosystem whose bacterial, viral, and fungal populations are imbalanced. The populations of beneficial bacteria that protect the health of your digestive system are reduced, and the populations of pathogenic bacteria and fungi (including Candida albicans) are flourishing. These pathogenic bacteria displace the beneficial bacteria that are so vital to digestion and absorption of food and maintaining a proper barrier between your gut and your blood. As the lining of the gut wall degrades, so do the cells that absorb nutrients from your food and that provide housing for the many digestive enzymes that break down your food, including lactase (the enzyme that breaks down lactose, milk sugar) and sucrase (the enzyme that breaks down sucrose, table sugar). Undigested carbohydrates like lactose, sucrose, and many complex starches are now being brought to the colon undigested where they feed the pathogenic bacteria and encourage their proliferation. As the population of pathogenic bacteria gets more and more out of control, the chemicals that they produce and the inflammation that they trigger begins to create openings between the cells of the intestinal wall, allowing undigested food particles (proteins in particular), bacteria, viruses, fungi, and bacterial toxins to move directly into the blood stream [28]. A vicious cycle has begun.

From there a host of diseases can arise, depending on what types of toxins, pathogens, and proteins get passed into the blood stream and begin to circulate throughout the body. Bacterial toxins in the blood can make it to the brain where they can cause severe neurological diseases such as autism, schizophrenia, and bipolar disorder. Bacteria, viruses, and fungi circulating through the blood trigger a systemic immune reaction, leading to chronic infection and inflammation. Undigested proteins (the most notorious being gluten and casein) mimic the structures of certain proteins in your tissues, and as your immune system works to eradicate the foreign protein from your system, it begins attacking your own tissue that looks a lot like that foreign protein. This is known as autoimmune disease, and there are many modern diseases that are autoimmune (and thus digestive) in origin, including type 1 diabetes, rheumatoid arthritis, and multiple sclerosis. As you can see, so many of the chronic diseases that are so prevalent in modern society and that were virtually non-existant in traditional cultures are the result of eating a toxic diet that we are not evolved for and the gut dysbiosis that comes as a consequence [28,29].

Evolutionarily, a carbohydrate-heavy diet is a newfangled cultural artifact introduced less than 10,000 years ago. Depending on your ethnic background, your ancestors may not have been eating grains for that long, in some cases only a few hundred years or less. That is certainly not a long enough time for our digestive systems to evolve enough to subsist on them. Starches in the form of root vegetables have been eaten since the Paleolithic period, but only in small quantities and as a supplement to a fat-, protein-, and green-vegetable-heavy diet. Our modern diets of the Western world are extremely heavy in carbohydrates, specifically sugars and starches, which are very difficult for our Paleolithic digestive systems to digest. If continued long enough, adherence to this carb-heavy diet (especially the Standard American Diet, a.k.a. SAD) will begin to feed pathogenic bacteria and fungi in the intestines that thrive on simple sugars and starches. Gut dysbiosis results, and when those imbalanced bacteria are passed between mother and child generation after generation, in addition to any negative dietary habits the parents have, the child develops leaky gut syndrome.

Not all carbohydrates are harmful for those with gut dysbiosis, leaky gut, or GAPS. There are carbs that our Paleolithic digestive systems have an easier time digesting, such as the monosaccharides (glucose, fructose, and galactose) and gentler fibers in fruits and non-starchy vegetables. Monosaccharides can be absorbed directly into the blood stream via the small intestine with no additional digestive action, providing energy and sustenance. The fiber in fruits and vegetables is different from that in grains and starchy vegetables (e.g. potatoes) and is easier for the system to digest. However, when first beginning the GAPS protocol, certain very fibrous vegetables (e.g. celery) should be avoided until the intestinal bacteria community rebalances itself and the pathogenic bacteria and fungi are reduced to some extent, because fiber can feed those pathogens if severe dysbiosis is still present. Complex sugars (disaccharides and polysaccharides) and grain fibers are much harder to break down and require the aid of digestive enzymes and/or culinary fermentation (e.g. souring grains) for complete digestion and absorption. For those with dysbiosis, this part of the digestion doesn't work properly or is not enough to get the job done. When complex sugars, starches, fibers, and grain brans do not get completely broken down in the upper part of the digestive tract they provide food for pathogenic bacteria in the colon where they release gases and toxins that cause inflammation, pain, and leaky gut.CF and Leaky Gut SyndromeLeaky gut syndrome, or increased intestinal permeability, is not a common term in the CF lexicon. But it should be, as more research is elucidating the importance of a healthy gut flora in CF as well as the hidden prevalence of leaky gut syndrome in the CF population. Research has shown that "the intestinal microflora of [CF] children is often abnormal due to massive exposure to antibiotics, and in addition their intestinal permeability is increased suggesting disruption of intestinal barrier function" and that "the disruption of the intestinal epithelial barrier is central to the pathogenesis of several inflammatory diseases. Interestingly, an increase in intestinal permeability has been reported in atopic dermatitis and IDDM, as well as in CF. These findings suggest that probiotics may contribute in several ways to the first line host defence to environmental challenges" [27]. In addition, research is finding that gut inflammation is very common in CF: "intestinal inflammation is another typical feature of CF and is much more common than previously thought. Recently, we reported that fecal calprotectin concentration and rectal nitric oxide production are increased in virtually all children with CF, suggesting that intestine is a target organ in CF and is constantly in an inflammatory state" [26]. As you have read, gut inflammation is a central part of the pathogenesis of leaky gut syndrome. Furthermore, maintaining a healthy gut microbiome via good diet and probiotic supplementation is essential to maintaining lung health: "our findings suggest that nutritional factors and gut colonization patterns are determinants of microbial development in the respiratory tract in infancy and present opportunities for early intervention in CF" [26]. From my communication with other CFers around the world, I've found that many of us are presenting typical symptoms of leaky gut syndrome (e.g. low IgA, high systemic inflammatory markers, Celiac's and Crohn's disease, chronic bloating and gas, achy joints and bones, food sensitivities, etc.) yet our docs are not fitting the pieces of the puzzle together. It is my belief that these symptoms are not atypical of CF. In the future, I believe modern medicine will be treating CF and leaky gut syndrome together, as two aspects of the same disease, and maintaining a healthy intestinal ecosystem will become a central facet of mainstream CF treatment. Let us hope the medical establishment is enlightened sooner rather than later.

Since essentially all CFers have some degree of carbohydrate malabsorption and leaky gut syndrome, the only way to fix this problem at its roots is to eliminate complex sugars and starches as well as other foods that exacerbate leaky gut, and to eat probiotics to restore a healthy gut flora. The Gut and Psychology Syndrome (GAPS) diet is the most comprehensive way to begin the healing process. I will explain more about the steps that need to be taken in the various other sections in my website.

Author

Mica is a clinical herbalist specializing in addressing issues related to cystic fibrosis, severe respiratory diseases, nutrition and digestion, diabetes and blood sugar disregulation, immune disregulation, and much more. Through their own personal experiences with chronic illness, they are passionate about empowering people to take charge of their own health with natural, holistic, and integrative approaches. Please ask questions or share what's worked for you!

*****************************Disclaimer: The content of this website and blog is for educational purposes only and should not be considered medical advice. I am not a licensed medical professional and do not take responsibility for any actions taken by the reader as a result of access to this information.